Elsevier

Oral Oncology

Volume 47, Issue 12, December 2011, Pages 1105-1109
Oral Oncology

Review
Treatment guidelines of lymphatic malformations of the head and neck

https://doi.org/10.1016/j.oraloncology.2011.08.001Get rights and content

Summary

Lymphatic malformations, traditionally called lymphangiomas, are diseases caused by development errors of the lymphatic system. About 90% of the cases occur within 2 years of age, except a few cases which occur in adulthood, and approximately 75% of the lesions are located in the head and neck region. The lesions can grow rapidly with infection, trauma or bleeding, resulting in disfigurement as well as severe impairment of respiration, swallow and speech. Although lymphatic malformations are benign lesions, they rarely resolve spontaneously, their infiltrating nature coupled with the difficulty in distinguishing involved vital structures of head and neck from adjacent normal tissues makes complete surgical resection even more difficult. The likelihood of postsurgical recurrence and complications is thus higher than other vascular lesions. Surgical resection, sclerotherapy and laser therapy are currently the main treatment modes of lymphatic malformations. Various treatment options have their advantages and disadvantages, the selection of treatment modalities should depend on the patient’s individual status and available technology and expertise. The treatment protocol should be individualized, comprehensive as well as sequential in order to obtain the best treatment outcome. Based on published literatures and clinical experiences, we devised the treatment guideline for management of head and neck lymphatic malformations. This protocol will be reviewed and updated periodically to include cutting edge knowledge to provide the best treatment options to benefit our patients.

Section snippets

Clinical and histopathological features

Most lymphatic malformations appear at birth, but the clinical symptoms often present at older ages. Gross6 reported that 50–75% can be diagnosed at birth, with 80–90% of the remaining cases diagnosed by the age of 2 years. Infection, trauma or changes in hormone level can result in destruction of lymphatic circulation and expansion of the lesions. Radiation therapy or connective tissue diseases (CTDs) can also aggravate the condition.

More than 70% of lymphatic malformations are found in the

Diagnosis and treatment

It is not difficult to make the diagnosis of LMs based on history and clinical manifestations. Lesions in oral mucosa commonly present as microcystic, isolated or multiple sporadic round nodules or punctuate lesions. The lesion is soft in texture and pink to dark red in color scattered with yellow vesicles, sometimes with venous malformations. The most commonly affected areas of deep-seated LMs are the lower 2/3 of the face such as the lip, cheek, tongue and ear, etc., which often cause

Treatment modalities

Many treatment methods have been documented in the literature, including surgery, various sclerotherapy and laser therapy. Localized microcystic lesions can be resected completely, but impossible for extensive and diffuse ones, the reasons being: (1) most of them involve the lip, cheek and tongue, complete excision may result in severe tissue defects, leading to cosmetic and functional complications; (2) the lesions are poorly demarcated; (3) the walls of lymphatic vessels of LMs are thin and

Choice of treatment methods

The choice of treatment should be individualized and based on several factors such as the hyoid level, bilaterality, age of onset, growth rate, type, depth, extent, anatomical location, potential deformity or dysfunction of LMs.19 Lesions with severe life-threatening functional impairment should be treated early. When there is no significant functional deficit, treatment can be delayed well past infancy and 18–24 months is available to allow for spontaneous resolution.[20], [21] Localized

Intralesional injection of Pingyangmycin

Bleomycin is a cytotoxic antitumor antibiotic that can be administered intralesionally by means of transcutaneous injection and is proving to be an exciting modulator of vascular anomalies.22 Pingyangmycin (PYM) is a single-component A5 of various components of bleomycin, because of the low cost, safety, and ease of availability, percutaneous intralesional injection of Pingyangmycin has been used more frequently for management of vascular malformations as a single modality or in combination

Laser therapy

Laser therapy has been used to treat superficial lymphatic malformations, especially for patients with localized infection. The advantage of laser therapy is the ease of use, less bleeding, minimal pain, reliable effect and repeatable treatment. Atropine can be used preoperatively to reduce salivary secretion. The wavelength of CO2 laser is 10,600 nm (far-infrared light) with 0.2 mm spot size and 0–6 W continuous output power. The laser probe should be kept at a distance of 0.5–1 cm from the

Combined therapy

Laser therapy and PYM sclerotherapy can be used alternatively or jointly for superficial microcystic lymphatic malformations of the tongue and oral mucosa. The local tissues may become thicker and harder after several injections. Local injection of triamcinolone acetonide can loosen and soften the tissues making the lesions thinner. It must be addressed that the pharmaceutic effect of triamcinolone acetonide could last 2 months, thus the dosage for children below 2 years old should be less then

Deep-seated microcystic lymphatic malformations

Treatment of deep-seated microcystic LMs is still a challenging problem; surgical resection alone usually has a poor outcome, often resulting in secondary deformities. Therefore, surgery is no longer the preferred method for deep-seated microcystic LMs. Intralesional injection of OK-432 or Pingyangmycin has been recommended with excellent result in some patients through repeated injections. For complicated cases, surgical correction may be applied after laser therapy and/or sclerotherapy to

Macrocystic lymphatic malformations

Sclerotherapy with Pingyangmycin or OK-432 is the mainstay of treatment for macrocystic lymphatic malformations, and surgery is usually used as complementary therapy.[14], [27] The lymphatic fluid in the cystic space should be aspirated as much as possible through 7-gauge needle prior to injection of Pingyangmycin (2 mg/mL) or OK-432. The amount of OK-432 (0.1 mg/10 mL) is the same as the aspirated fluid, not more than 20 mL. Large macrocystic lymphatic malformations localized in the upper neck or

Conclusion

A number of treatment methods are available for lymphatic malformations of the head and neck region. Sclerotherapy has been most effective in the management of macrocystic LM. Superficial and localized lesions can be treated with intralesional injection and laser therapy, often with good results. If lesions are too extensive for complete excision, sclerotherapy may be used as the primary treatment of choice or postoperative adjunctive treatment. The use of laser therapy and sclerotherapy is

Conflict of interest statement

None declared.

Acknowledgment

We are very grateful to Dr. Kee Hau Wong from ParkwayHealth Shanghai for his careful revision of the manuscripts and valuable suggestions.

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